Lec 12 management of rheumatic fever rheumatic heart disease for mohs

Rheumatic Heart Disease

1. Management of Rheumatic Fever & Rheumatic Heart Disease Department of Cardiology Yangon General Hospital

2. Rheumatic Fever (RF) & Rheumatic Heart Disease (RHD) Pharyngeal infection with Lancefield group A ß-haemolytic streptococci triggers rheumatic fever 2-4 weeks later. Rheumatic Heart Disease - Non-suppurative complications of Group A streptococcal pharyngitis due to a delayed immune response

3. Rheumatic Fever (RF) & Rheumatic Heart Disease (RHD) Peak incidence : 5-15 yrs True infection (rising antibody response) or carrier state (no rising antibody) True infection : at risk of developing RF and of spreading the organism to close contacts Socioeconomic & environmental factors play an indirect but important role in the magnitude and severity of RF & RHD

4. Diagnostic criteria for rheumatic fever – modified 2015 Jones criteria ; Major criteria Low risk population Carditis (clinical or subclinical) Arthritis – only polyarthritis Chorea Erythema marginatum Subcutaneous nodules High risk population Carditis (clinical or subclinical) Arthritis – monoarthritis or polyarthritis Polyarthralgia Chorea Erythema marginatum Subcutaneous nodules

5. Diagnostic criteria for rheumatic fever – modified 2015 Jones criteria ; Minor criteria Low risk population Polyarthralgia Hyperpyrexia (≥ 38.5ºC) ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl Prolonged PR interval (after taking into account the differences related to age; if there is no carditis as a major criterion) High risk population Monoarthralgia Hyperpyrexia (≥ 38.0ºC) ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dl Prolonged PR interval (after taking into account the differences related to age; if there is no carditis as a major criterion)

6. Diagnosis of RF First episode of the disease – Two major criteria or one major and two minor criteria with evidence of antecedent group A β-hemolytic streptococcal infection Subsequent episodes - Two major criteria or one major and two minor criteria or three minor criteria

7. Clinical features of Rheumatic Carditis Pericarditis : (in primary episode or recurrence of RF) -rub supported by echo evidence of PE and simultaneous valvular involvement Myocarditis : unexplained CHF or cardiomegaly, almost always associated with valvular involvement. RHD patients – CHF, minor criteria, ↑ASO provide Rh: carditis Endocarditis/ valvulitis : Apical PSM ± MDM (Carey Coombs), basal EDM in pt: who do not have RHD Pt: with previous RHD, change in the character of murmur or the appearance of a new significant murmur indicates the presence of carditis Role of Echo in diagnosis of carditis is essential

8. Medical Management of RF General: Hospital admission- to confirm a diagnosis Bed rest – to monitor closely for the onset of carditis Rest period at least 4 weeks for carditis Investigations: throat culture, ASO, acute phase reactants; ESR, CRP, CXR, ECG, Echo, blood culture to exclude IE Antimicrobial Tx: Eradication of the pharyngeal strept: infection Two throat cultures before starting A/B

9. Medical Management of RF Suppression of the inflammatory process Should avoid premature administration of salicylates Aspirin 100 mg/kg/day divided into 4-5 doses (125 mg/kg/day in children) for adequate response but avoid toxicity Reduce to 60-70 mg/kg/day for 3-6 weeks Naproxen 10-20 mg/kg/day if intolerant or allergic to aspirin Corticosteroids: Not respond to Aspirin or for pericarditis or HF Prednisolone 2 mg/kg/day (80 mg/day) or methylprednisolone 2-3 wk, overlap with aspirin

10. Medical Management of RF HF in RF: bed rest, steroids, if severe symptoms, Diuretics, ACEI, digoxin For chorea: self-limiting benign disease, no threapy, Neuroleptics, benzodiazepines, anti-epileptics (Haloperidol, Diazepam, Carbamazepine ) Steroids are not beneficial for chorea

11. Primary prevention of RF: Recommended treatment for Streptococcal pharyngitis Phenoxymethyl penicillin > 40 kg – 2–3 MIU/day < 40 kg – 100,000 to 200,000 IU/kg/day PO in 2 divided doses every 12 hours for 10 days Benzylpenicillin >40 kg – 1.2 MIU < 40 kg – 600,000 IU. intramuscularly at a single dose Cefadroxil > 40 kg – 1 g < 40 kg – 30 mg/k hypersensitivity to penicillin single dose for 10 days Cefalexin adults 500 mg BD children 25– 50 mg/kg/day in 2 doses hypersensitivity to penicillin for 10 days Erythromycin > 40 kg – 0.2–0.4 g < 40 kg – 30–50 mg/kg/day every 6–8 hours for 10 days Clarithromycin > 40 kg – 250–500 mg every 12 hours < 40 kg – 15 mg/kg/day in 2 doses 10 days Azithromycin > 40 kg – 500 mg on the first day, then 250 mg for three consecutive days

12. Secondary prevention of RF Secondary prevention - prevention of subsequent rheumatic fever relapses Duration of secondary prevention - determined individually From 5 to 10 years from the last RF relapse, or up to 21 years of age (whichever is longer )

13. Suggested Duration of Secondary Prophylaxis Patients without proven carditis For 5 yrs after the last attack or until 18 yrs of age (whichever is longer) Patients with carditis (mild MR or healed carditis) For 10 yrs after the last attack or at least until 25 yrs of age (whichever is longer) More severe valvular disease Lifelong After valve surgery Lifelong

14. Antibiotics used in secondary prophylaxis of RF Benzathine benzylpenicillin 1 200 000 units, IM, 3-4 weeks 600 000 units for children Penicillin V 250 mg BD Sulphonamide e.g sulphadoxine, sulphadiazine 1 gm daily 500 mg daily for children Erythromycin 250 mg BD

15. Surgical referrals or percutaneous valvotomies Chronic rheumatic valve disease Determined by the severity of patient’s symptoms and significantly impaired cardiac function To prevent irreversible damage to the LV and irreversible pulmonary hypertension Echo is essential for an assessment and follow up of valvular disease

16. Referrals for further assessment > NYHA Class II. Note: with AS, all symptomatic patients Progressive LV enlargement on clinical or CXR Cardiac failure not due to episode of rheumatic carditis PHT with clinical signs and ECG evidence of RVH, and CXR evidence of pulmonary artery dilatation TR complicates mitral valve disease Development of AF Thromboembolism Endocarditis is suspected to contribute to cardiac decompensation

17. Treatment Options Balloon valvotomy (commissurotomy ) Surgical treatment Closed mitral commissurotomy Valve repair Valve replacement

18. Management of heart failure with rheumatic valvular heart disease Investigations: Baseline blood tests : Haemoglobin, electrolytes, creatinine, liver function test ECG CXR Assessment of rheumatic activity

19. Management of heart failure with rheumatic valvular heart disease Life style modification: restriction of salt intake Avoidance of precipitating factors Diuretics: Frusemide, Spironolactone, Metolazone Heart rate control : digoxin, beta blocker Anticoagulation: warfarin Therapeutic INR  2 to 2.5 Metallic valve : Singe valve  2 to 2.5 Metallic valve : Double valve  2.5 to 3 Prophylaxis : Penicillin

20. ACEI/ ARB – recommended only for non rheumatic valvular regurgitations Patients should be referred to tertiary centres for assessment of valvuloplasty or valve replacement Management of heart failure with rheumatic valvular heart disease

21. MITRAL STENOSIS

22. Medical management Salt intake restriction and oral diuretics In AF: digoxin, B-blocker, or calcium-channel blocker for rate control. Anticoagulation Endocarditis prophylaxis is no longer recommended Balloon valvotomy Surgical treatment - Valvotomy, Mitral repair, MVR

23. MITRAL REGURGITATION

24. Medical management Asymptomati mild MR are managed conservatively with serial echocardiograms. Vasodilators in symptomatic patients to increase forward CO and reduce regurgitant volume. AF: rate control and anticoagulation Surgical treatment Symptomatic severe MR on optimum medical management. Asymptomatic patients with severe MR may need surgery if worsening of LV function (EF 30–60 % or LV end-systolic dimension > 40 mm), new AF , pulmonary hypertension.

25. AORTIC STENOSIS

26. Medical management No medical treatments are proven to prevent or delay the disease process in the AV leaflets. B -blockers reduce myocardial O 2 demand and may improve coronary blood fl ow Cautious use of loop diuretics may relieve preload and help with dyspnoea (avoid hypovolaemia) In CHF or dilated LV, digoxin may help with dyspnoea (particularly if patient is in AF/fl utter) In severe AS, avoid negative inotropes and drugs that reduce afterload (e.g. glyceryl trinitrate (GTN,) angiotensin-converting enzyme inhibitors (ACE-Is)), as these may worsen the gradient and cause syncope.

27. Surgical treatment Symptomatic AS. In asymptomatic severe AS, EF<50 % Aortic valve replacement (AVR) is reasonable for asymptomatic moderate or severe AS when undergoing concomitant coronary artery bypass graft (CABG), aortic, or valve surgery . Balloon aortic valvuloplasty Transcatheter aortic valve replacement

28. AORTIC REGURGITATION

29. Medical management Asymptomatic mild/moderate AR with normal LV — routine follow-up (every 1–2 years) with ECHO. Asymptomatic severe AR with normal LV — frequent (6-monthly) follow-up Severe AR with LV dysfunction or symptoms (and patient not operative candidate) — symptoms of CCF respond to loop diuretics and digoxin and vasodilators (ACE-Is, calcium-channel blockers) Surgical management AVR is indicated for patients with symptomatic severe AR, or asymptomatic severe AR and EF < 50 % , severe LV dilatation (LV end-diastolic dimension > 75 mm or LV end-systolic dimension > 55 mm), or concomitant CABG, valvular, or aortic surgery.

30. Tricuspid and pulmonary disease Tricuspid regurgitation Functional or secondary TR Organic tricuspid lesions: endocarditis, transvalvular pacing wires, Marfan’s syndrome, Ebstein anomaly, rheumatic heart disease, carcinoid. In the absence of pulmonary hypertension, TR is well tolerated and may not require specifi c treatment TV annuloplasty Valve replacement Tricuspid stenosis Extremely rare surgical valvuloplasty/ replacement

31. Pulmonic stenosis Balloon valvuloplasty — treatment of choice for stenosis at valvular level. Surgical – valvulotomy , Pulmonic valve replacement Pulmonary regurgitation Supportive treatment

32. Prosthetic heart valves Mechanical: Bileaflet (St Jude Medical ® , Carbomedics ® ) most common today. Ball and cage (Starr–Edwards ® ) or tilting disc (Medtronic Hall ® ) Bioprosthetic: Porcine (stented or stentless) or bovine pericardium (Carpentier–Edwards) Non prosthetic valves: Homograft (preserved cadaveric human valve), autograft (pulmonary valve — Ross procedure ) Anticoagulation ( target INR )

33. Planning & implementation of national programs for the prevention & control of RF & RHD A strong commitment at policy level ( Ministries of H&E) A national advisory committee: Cardiologists, paediatricians, family physicians, internal medicine specialists, epidemiologists and nurses Stepwise program implementation: one or more defined local areas (phase I) to provincial (phase II) & national coverage (phase III) Service orientated and emphasize active secondary prevention, integrated into PHC Support from the microbiology lab at peripheral, intermediate and national levels Suspected outbreaks of gp A strept: infection should be controlled and studied

34. Main Components of A National Program Secondary prevention activities aimed at preventing the recurrence of acute RF and severe RHD (case finding, referral, registration, surveillance, follow-up, secondary prophylaxis) Primary prevention activities aimed at preventing the first attack of acute RF (early detection, correct dx, appropriate tx of strept: pharyngitis) Health education activities (P&S prevention, recognizing and reporting sore throats) Training of health care providers Epidemiological surveillance Community involvement

35. THE END